Department of Gynecological Surgery and Gynecological Oncology of Adults and Adolescents, Pomeranian Medical University in Szczecin, al. Powstańców Wielkopolskich 72, 70-111 Szczecin, Poland.
Department of Nephrology, Transplantology and Internal Medicine, Pomeranian Medical University in Szczecin, al. Powstańców Wielkopolskich 72, 70-111 Szczecin, Poland.
Int J Environ Res Public Health. 2022 Oct 25;19(21):13862. doi: 10.3390/ijerph192113862.
Granulomatosis with polyangiitis is a rare autoimmune disease with the presence of c-ANCA in most cases. It involves necrotizing inflammation in small and medium-sized vessels with multiple granulomas. The disease can affect many systems, but the typical triad of attacked systems are the upper and lower respiratory tracts and kidneys, with varying degrees of severity. Involvement of the respiratory tract may manifest, among other symptoms, as nasal crusting, nosebleeds, and dyspnea. Among patients with granulomatosis with polyangiitis, only less than 1% develop genitourinary system involvement. We present a case study of a 36-year-old woman with an 8-year-long GPA history and a lesion, which, due to its appearance and accompanying symptoms, aroused the suspicion of a neoplasm but was proven to be a granuloma with a nontypical location. The systemic disease was treated with glucocorticosteroids and cyclophosphamide. The lesion on the labium minus was surgically removed. We concluded that the macroscopic picture of GPA of the vulva and vulvar cancer is similar. The patient's medical history may help differentiate GPA and vulvar cancer. Although vulvar GPA is extremely rare, it should be considered in the differential diagnosis of vulvar lesions, especially those suspected to be oncological.
肉芽肿性多血管炎是一种罕见的自身免疫性疾病，大多数情况下存在 c-ANCA。它涉及小血管和中等大小血管的坏死性炎症和多个肉芽肿。这种疾病可以影响许多系统，但典型的三联征是上呼吸道、下呼吸道和肾脏，严重程度不一。呼吸道受累可能表现为鼻腔结痂、鼻出血和呼吸困难等症状。在肉芽肿性多血管炎患者中，只有不到 1%的患者出现泌尿系统受累。我们报告了一例 36 岁女性患者，她患有 GPA 病史 8 年，病变由于其外观和伴随症状引起了肿瘤的怀疑，但被证实是非典型部位的肉芽肿。全身疾病用糖皮质激素和环磷酰胺治疗。小阴唇上的病变通过手术切除。我们得出结论，外阴 GPA 的大体表现与外阴癌相似。患者的病史可能有助于区分 GPA 和外阴癌。尽管外阴 GPA 极为罕见，但在外阴病变的鉴别诊断中应考虑到它，特别是那些疑似为肿瘤性的病变。
